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Abstract: FR-PO1125

Adult Outcome of Childhood-Onset Steroid-Dependent Idiopathic Nephrotic Syndrome in the Rituximab Era

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Plaisier, Emmanuelle M., APHP Tenon, Paris, France
  • Dossier, Claire, APHP Robert Debré, Paris, France
  • Dahan, Karine, APHP Tenon, Paris, France
  • Ronco, Pierre M., APHP Tenon, Paris, France
  • Baudouin, Veronique, APHP-Robert Debré Hospital, Paris, France
  • Ulinski, Tim, APHP - Trousseau and UPMC, Paris, France
  • Deschênes, Georges, APHP Robert Debré, Paris, France
Background

Idiopathic nephrotic syndrome (INS) represents the most frequent glomerular disease in childhood, with up to 60% of patients experiencing frequent relapses and steroid dependency requiring long-lasting steroid therapy and immunosuppressive (IS) drugs. Outcome and optimal therapy at adulthood, as well as long-term complications of childhood IS exposure is poorly documented.

Methods

We retrospectively analyzed a monocentric cohort of 43 patients with childhood-onset steroid-dependent INS. Relapses in adulthood were treated with steroids or a combinaison of steroids and rituximab (RTX), with the exclusion of all other IS .

Results

Median age at the INS diagnosis and at the last follow-up was 4 years (1-17) and 24 (19-40) years, respectively, with a median duration of follow-up at adulthood of 7 (1-21) years. At pediatric age, 42 (98%) patients received at least one nonsteroidal IS drug, including calcineurin inhibitors in 29 (67%), mycophenolate mofetil in 26 (60%), RTX in 17 (39%), an alkylating agent in 15 (35%), and levamisole in 13 (30%). At entry in the adult care, 16 (37%) of the patients still received an IS drug other than RTX. Relapse occurred in 32 (74%) of patients at adult age with 5 patients presenting at least 2 episodes. In 9 patients, relapse occurred more than 3 years after steroids and IS discontinuation. Relapses were treated with steroids alone in two patients, while the other 29 received a combination of steroids and RTX, followed, for 20/29 patients, by a reinfusion of RTX either at B-cell recovery or at fixed intervals (6 to 12 months), up to 24 months. At last follow-up, 36 (84%) patients were in complete remission, with a treatment-free duration of more than 24 months in 12 (28%), of 12 to 24 months in 8 (19%) and of less than 12 months in 16 (37%). All but one patients were free of steroids and IS drugs. No patient experienced infection or cytopenia and one patient developed neoplasia most likely unrelated to IS.

Conclusion

Childhood-onset steroid-dependent INS remains a concern at adulthood with a high rate of relapse occurring after a variable delay. RTX is a safe and efficient steroid-sparing agent allowing maintenance of remission and discontinuation other IS. No late-onset severe complication related to childhood therapies was recorded.